Hereditary Fructose Intolerance

Acceptability at Recruitment

UNACCEPTABLE

Acceptability at VT / Work-Up

UNACCEPTABLE

Individual at risk

Donor

Explanation of Condition

Hereditary Fructose Intolerance (HFI) is a disorder in which a person lacks the protein needed to break down fructose. This condition occurs when the body is missing an enzyme called aldolase B. This substance is needed to break down fructose. If a person with HFI eats fructose or sucrose, complicated chemical changes occur in the body. The body cannot change its stored form of sugar (glycogen) into glucose. As a result, blood sugar falls and dangerous substances build up in the liver. HFI requires a lifelong avoidance of fructose, sucrose, and sorbitol. These sugars are commonly found in many foods and medications, and even small amounts can trigger severe reactions. HFI can lead to liver damage, kidney problems, and other health issues. Although HFI is not directly transmissible, the recipient of the bone marrow transplant will be immunocompromised and vulnerable to complications from the donor's dietary restrictions and potential underlying health issues.

Guidance

People with HFI must not be given Zarzio or Accofil (filgrastim) as they contain fructose/sorbitol (E420).

Not acceptable under any circumstances.

Pseudonyms or Related Conditions

Fructosemia

Fructose intolerance

Fructose aldolase B-deficiency

Fructose-1

6-bisphosphate aldolase deficiency

HFI

Version

Version 1, Edition 1

References

1) https://www.medicines.org.uk/emc/product/6672/smpc#PRECLINICAL_SAFETY

2) https://www.ema.europa.eu/en/documents/product-information/zarzio-epar-product-information_en.pdf

3) https://medlineplus.gov/ency/article/000359.htm

4) Accofil, INN-filgrastim